Renal lymphangiectasia is an unusual benign anomaly. The case of a 34-year-old woman with bilateral renal lymphangiectasia is presented in this report. The nomenclature of this disease is confused in the literature. The possible aetiology of renal lymphangiectasia is discussed, along with its clinical manifestation, characteristics on ultrasonography, computed tomography and intravenous urography. The disease evolves slowly and usually needs no surgical treatment unless in an emergency.